Determining the Risk Factors for Anastomotic Stricture Development after Esophageal Atresia Repair: Results from the Turkish Esophageal Atresia Registry.

INTRODUCTION: Anastomotic stricture (AS) is the second most common complication after esophageal atresia (EA) repair. We aimed to evaluate the data in the Turkish Esophageal Atresia Registry to determine the risk factors for AS development after EA repair in a large national cohort of patients. METHODS: The data between 2015 and 2021 were evaluated. Patients were enrolled into two groups according to the occurrence of AS. Patients with AS (AS group) and without AS (non-AS group) were compared according to demographic and operative features, postoperative intubation status, and postoperative complications, such as anastomotic leaks, fistula recanalization, and the presence of gastroesophageal reflux (GER). A multivariable logistic regression analysis was performed to define the risk factors for the development of AS after EA repair. RESULTS: Among the 713 cases, 144 patients (20.19%) were enrolled into the AS group and 569 (79.81%) in the non-AS group. The multivariable logistic regression showed that, being a term baby (odds ratio [OR]: 1.706; p = 0.006), having a birth weight over 2,500 g (OR: 1.72; p = 0.006), presence of GER (OR: 5.267; p < 0.001), or having a recurrent tracheoesophageal fistula (TEF, OR: 4.363; p = 0.006) were the risk factors for the development of AS. CONCLUSIONS: The results of our national registry demonstrate that 20% of EA patients developed AS within their first year of life. In patients with early primary anastomosis, birth weight greater than 2,500 g and presence of GER were risk factors for developing AS. When patients with delayed anastomosis were included, in addition to the previous risk factors, being a term baby, and having recurrent TEF also became risk factors. LEVEL OF EVIDENCE: III.

Gastrointestinal Tract Duplications in Children: A Tertiary Referral Center Experience.

Objective: Gastrointestinal duplications are rare congenital anomalies. Herein, we present a single institutional experience in pediatric gastrointestinal tract duplications. Methods: Patient records from 2014 to 2019 were retrospectively evaluated for demographic data, clinical presentation, diagnostic methods, surgical findings, and pathological reports. Results: This study included 19 patients, of whom 10 were males and nine were females, with a median age of 30 (21 days-15.5 years) months. Three patients were antenatally and three were incidentally diagnosed. Abdominal pain, vomiting, constipation, and perianal accessory orifice were the most common presenting symptoms. Preoperative diagnostic workup included ultrasonography (n=13), cross-sectional imaging (n=8), and nuclear scintigraphy (n=1). A preoperative diagnosis was possible in 14 (74%) patients. The duplications originated from the foregut in seven (37%) patients, midgut in seven (37%), and hindgut in five (26%). Cystic duplications were observed in 14 (74%) patients and tubular in five (26%). The total surgical excision with (n=8) or without (n=10) associated organ resection was possible in 18 patients. Partial cyst excision with a complete mucosal removal was done in 1 patient. Heterotopic mucosa was present in six (32%) specimens. The respiratory origin with thyroid transcription factor-1 positivity was contained in two para-esophageal duplications. Among five patients with heterotopic gastric mucosa, 1 had presented with perforation and the others with hemorrhage. Conclusions: Duplications may involve any gastrointestinal segment. The clinical presentation is highly variable because of the wide variation in the involved segment and sizes and the possibility of bearing heterotopic mucosa. The surgery aims to totally excise the cyst or at least totally remove the inner mucosal lining.

Feasibility, Reliability, and Validity of the Turkish Version of the Esophageal-Atresia-Quality-of-Life Questionnaires to Assess Condition-Specific Quality of Life in Children and Adolescents Born with Esophageal Atresia.

BACKGROUND: This study reports the feasibility, validity and reliability of the Turkish versions of the Esophageal-Atresia-Quality-of-Life (EA-QOL) questionnaires, which were originally developed in Sweden and Germany. METHODS: After translation from Swedish to Turkish and cognitive debriefings, 51 families of children aged 2 to 7 years (parent-report, 17-items) and 54 families of children 8 to 17 years (child and parent-report, 24-items) responded to the EA-QOL questionnaires and a validated generic HRQOL-instrument (PedsQL4.0). Medical records of patients and questionnaires were used to obtain clinical data. The Turkish version of the EA-QOL questionnaires were evaluated for feasibility (<5% missing item responses), reliability (internal consistency/retest reliability for 3 weeks) and validity (known-groups/concurrent/convergent). Level of significance was p<0.05. RESULTS: Feasibility of the Turkish version of the EA-QOL questionnaires was good. Internal consistency of all scales was satisfactory, as were the level of agreements of EA-QOL scores between the field- and retest study. Known-group and concurrent validity were achieved since the EA-QOL questionnaires showed that esophageal symptoms and feeding difficulties were negatively associated with EA-QOL total scores in both age specific versions (child-and parent report), and respiratory symptoms in the version for EA children 2 to 7 years (parent-report). A higher number of respiratory symptoms decreased the EA-QOL total scores in both age groups (parent-report, p<0.05). Correlations between the EA-QOL total scores and PedsQL-4.0 total scores supported convergent validity. CONCLUSION: The Turkish version of the EA-QOL questionnaires are feasible, valid and reliable to assess condition-specific HRQOL in EA children.

Calretinin immunohistochemical staining in Hirschsprung's disease: An institutional experience.

Objective: This study aims to evaluate the results obtained by calretinin staining on tissue samples for diagnosing Hirschsprung's disease (HD) in a single institution, by single expert. Methods: A retrospective evaluation was done for calretinin immunostaining in HD patients for a period of 3 years. Calretinin staining was evaluated in nerve fibers. Calretinin immunohistochemistry was considered positive if any staining was seen in nerve fibers and/or ganglion cells in the lamina propria, muscularis mucosa or submucosa. According to staining intensity, staining was classified as strong, weak or negative. The pathological diagnosis was based on presence or absence of ganglion cells (G0/G1) and nerve hypertrophy (N0/N1). Samples were classified according to the depth (presence of submucosa or intermuscular area), the type (biopsy or resection specimen) and staining intensity of calretinin (strong, weak, or negative staining). Results: A total of 96 tissue samples from 56 patients were studied. Tissues were from colon (43.8%), rectum (43.8%), stoma (6.2%), ileum (3.1%) and appendix (3.1%). The pathological diagnosis was G0N0 in 14.6%, G1N0 in 54.2%, G0N1 in 25% and G1N1 in 6.2% of cases. Our materials consisted of 92 tissue biopsies and four resection specimens. Intermuscular layer was present in 87.5% of materials and 12.5% of biopsies contained submucosa. Calretinin staining was negative (C0) in 37.5% of cases, strong positive (C1) in 47.9%, and weak positive (C2) in 14.6%. When the C0 category was taken as the reference, the status of calretinin staining as C2 (weak positive) in cases with pathological diagnosis of G1N0 was found to be 37.575 times that of cases with G0N0 (OR [95% CI]: 37.575 [2.928, 482.176], p=0.006) and the status of calretinin staining as C1 (strong positive) in cases with pathologic diagnosis of G1N0 was found to be 131.401 times that of G0N0 (OR [95% CI]: 131.401 [9.263, 1864.082), p<0.001). Conclusion: Calretinin staining is positive whenever ganglion cells are present independent from presence of nerve hypertrophy, the depth and the site of the biopsy or staining intensity. It is negative in all aganglionic samples. Calretinin staining is a reliable ancillary test in HD diagnosis.

Epiphrenic Diverticulum in an Infant with Congenital Esophageal Stenosis Associated with Esophageal Atresia.

Congenital esophageal stenosis (CES) is a rare congenital disorder which may be isolated or associated with esophageal atresia (EA). It courses with esophageal outflow tract obstruction. Esophageal epiphrenic diverticulae are esophageal outpouchings above diaphragm which develop because of an underlying esophageal motor disorder. We present an infant who had CES associated with EA detected during the course of routine follow-up. She underwent several sessions of esophageal balloon dilatations for CES with some symptomatic improvement. However, an epiphrenic diverticulum (ED) developed during the course of treatment which was detected by further investigations. A surgical excision was performed with a successful outcome. An ED developed secondary to CES has not been previously reported. We have highlighted the diagnostic modalities employed, treatment options, and clinical follow-up for these two rare conditions in children.

Mitotically active plexiform fibrohistiocytic tumor.

Plexiform fibrohistiocytic tumor is an intermediate malignant tumor situated in superficial soft tissues. It affects children and young adults. The tumor is most commonly located on upper extremities, whereas involvement of back region is rare. Mitotic activity is generally low (~3/10 HPF). It is rare, but it can exhibit aggressive behavior, so total excision with clear surgical margins and long-term followup is necessary to detect local recurrence and metastases. We report a child with a solid mass on back region which was found to be a mitotically active plexiform fibrohistiocytic tumor (6/10 HPF) after excision. Plexiform fibrohistiocytic tumor (PFT) is a mesenchymal neoplasm of children, adolescents, and young adults. It is characterized by fibrohistiocytic cytomorphology and multinodular growth pattern. Clinically it is usually a slow-growing mass of upper extremities with frequent local recurrence and rare regional lymphatic and systemic metastasis (Fletcher et al. (2002), Enzinger and Zhang (1988), Remstein et al. (1999)).

An evaluation of individual plain abdominal radiography findings in pediatric appendicitis: results from a series of 424 children.

BACKGROUND: This study was conducted to collect the results of the plain abdominal radiography (PAR) signs, to determine their individual diagnostic values, and to discuss them under a brief literature review. METHODS: Eight predetermined PAR signs were individually interpreted in a series of 424 consecutive children (278 males; 146 females; median age 10 years; range 11 months to 17 years) who underwent an operation for appendicitis. The sensitivity, specificity, and positive and negative predictive values for the signs were determined. RESULTS: Appendicitis was confirmed in 378 (89%) patients. Among the remaining 46 (11%) patients with a normal appendix, 20 (5%) had other intraabdominal pathologies. Calcified fecalith, mass image in right-lower-quadrant (RLQ), psoas obscuration, and localized extraluminal air signs were all highly specific and therefore, unlikely to be present if the appendix is normal. On the other hand, the sensitivity values were low, in general, for all the PAR signs investigated. Yet, presence of dilated transverse colon and/or single air fluid level in the RLQ has the highest percentage occurrence with appendicitis. CONCLUSION: Although the there is no single PAR finding capable of ruling the diagnosis of appendicitis out, basic knowledge on PAR findings could have an impact on decision making process for clinicians dealing with pediatric acute abdominal pain.